Beckwith-Wiedemann syndrome, delayed abdominal wall closure, and neonatal intussusception--case report and literature review.

We present the extremely rare case of a male newborn with Beckwith-Wiedemann Syndrome (BWS) presenting as delayed abdominal wall closure and neonatal intussusception. Fetal ultrasound had shown omphalocele that resolved spontaneously. When feeding was attempted, he had various episodes of vomiting. An x-ray showed signs of high bowel obstruction. Jejunal intussusception was found on laparotomy. Enterectomy and primary jejuno-jejunal anastomosis was performed. During post-operative period subtle physical findings became prominent: plain hemangioma, posterior helical indentations, and macroglossia. Cardiac ultrasonography showed a patent foramen oval with small left-to-right shunt. Ultrasonography showed renal hyperplasia. Genetic study showed hypomethylation of DMR2 region of 11p15 chromosome.

Effect of laparoscopic adjustable gastric banding on modifiable cardiovascular risk factors in extremely obese adolescents.

Recently, surgical intervention has gained increasing support in adolescents with extreme obesity. This study summarizes the analysis into the effect of laparoscopic adjustable gastric bands (LAGB) on cardiovascular risk factors in 14 extremely obese Portuguese adolescent patients. Data collected both pre- and postoperatively included age, gender, body mass index (BMI), percentage of excess weight loss, cardiovascular risk factors, and cardiovascular outcomes. Ten girls and four boys aged from 13.5 to 17.5 years underwent LABG. The mean preoperative weight and BMI were 127.4 kg and 46.1 kg/m(2), respectively. The average percentage of weight loss calculated was 32 % at 1 year, 38.8 % at 2 years, and 48.1 % at 3 years of follow-up. Simultaneously, blood pressure and insulin resistance index returned to normal, and there was an increase in high-density lipoprotein levels, 3 years after the LABG was fitted. LABG fitting is a safe and effective treatment strategy for the improvement of cardiovascular status following weight loss.

Upper pole nephrectomy: a simplified technique using a retroperitoneal laparoscopic approach.

Objective. To describe a simplified technique already used in our institution for several years in the open heminephrectomy for duplication anomalies, now performed through a retroperitoneal laparoscopic approach. Methods. The technique begins with upper pole parenchyma incision since the demarcation between the affected upper moiety and the healthy lower pole is easily established. The dissection proceeds until the urothelium of the collecting system is entered, which will guide further excision, minimizing damage of the surrounding structures. The vascular supply is then identified since the upper pole is attached to the remaining renal parenchyma only by these structures that can be safely divided. Dissection and division of the ectopic ureter is carried next. Results. The operative time was 188 minutes. The blood loss was not significant, and there were no other complications during the procedure. The patient was discharged home 48 hours after the procedure, without any early or late postoperative complications. Conclusion. We believe this simplified technique allows a safer excision of nonfunctioning upper pole renal tissue by avoiding the initial dissection of the renal hilum, which associated with the known advantages of a laparoscopic approach makes us consider it the procedure of choice for upper pole nephrectomy in children.

[Retroperitoneal laparoscopic nephrectomy in children younger than nine years-old: state of the art]. / Nefrectomia laparoscópica retroperitoneal em crianças com idade inferior a nove anos: estado da arte.

INTRODUCTION AND AIM: In Portugal, there is very few experience in pediatric retroperitoneoscopy. The authors present the first Portuguese series of retroperitoneal laparoscopic nephrectomies (RLN) in children younger than nine years-old, as well as a literature review about the theme. MATERIAL AND METHODS: Retrospective analysis of clinical charts of all children submitted to RLN between January 2009 and December 2009 in a Pediatric Surgery Department. The literature review was made searching related articles in Medline. RESULTS: Eight RLN were preformed. The medium age of the patients was 4,5 years-old (minimum = 11 months, maximum = 8,6 years). The indications for surgery were: four multicystic kidneys, three reflux nephropathy, and one obstructive nephropathy. Four RLN were preformed on the left side. The medium operative time was 99 minutes (minimum = 50 minutes, maximum = 180 minutes). There was a shortening of operative time as the surgical team got more experienced. There were no conversions to open surgery. Medium hospital stay was 1,5 days (minimum = 1 day, maximum = 2 days). There were no intra-operative and no post-operative complications. CONCLUSIONS: RLN is feasible in children younger than nine years-old and should be considered standard treatment in pediatric population.

Varicocoelectomy in adolescents: laparoscopic versus open high ligation technique.

BACKGROUND: Treatment of varicocoele is aimed at eliminating the retrograde reflux of venous blood through the internal spermatic veins. The purpose of this investigation was to compare laparoscopic varicocoelectomy (LV) with open high ligation technique in the adolescent population. MATERIALS AND METHODS: We retrospectively evaluated 33 adolescents who underwent varicocoelectomy at our paediatric hospital, between May 2004 and September 2008. Patients were divided into two groups depending on the technique: those who had an LV and those submitted to an open varicocoelectomy (OV). We analysed side, age of surgery, follow-up period and the incidence of recurrence/persistence, hydrocoele formation and wound complication. RESULTS: There were 24 patients in the LV group and 9 in the OV group. All varicocoeles were in the left side. Mean age was 12 years in both groups. Mean follow-up time was 32 months for the LV group and 38 months for the OV group (P = 0.49). There was no significant difference in the incidence of hydrocoele in both the groups (25% versus 22%, P = 0.626). There was no recurrence/persistence on the LV group, while in the OV group there were three cases (P = 0.015). CONCLUSION: LV seems more efficient than open high ligation technique in the treatment of adolescents' varicocoeles. Larger series are necessary to draw more reliable conclusions.